Gastrostomy and mechanical ventilation in amyotrophic lateral sclerosis: how best to support the decision-making process?

Barć, Krzysztof; Kuźma‐Kozakiewicz, Magdalena

doi:10.5603/pjnns.a2020.0081

Cited by 13 publications

(20 citation statements)

References 118 publications

(223 reference statements)

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“…The estimated depressiveness, ranging from moderate to high degree, was found to be higher in the Polish group. This general attitude of the physicians does not mirror the widely reported maintenance of good QoL in PALS following the start of PEG or NIV 7 . Notably, in a recent study of PALS in a locked‐in state (n = 17 on IV, n = 2 on NIV), most interviewed individuals reported a positive QoL and no signs of depression 21 .…”

Section: Discussionmentioning

confidence: 88%

“…Overall, the estimated depressiveness (eDep) was of moderate value for patients using PEG or IV (5 and 5, respectively) and of high value for those using IV 7 . The Polish group reported significantly higher values of eDep in individuals using PEG (6 vs. 4, p < 0.001), NIV (7 vs. 4, p < 0.001) and IV (8.5 vs. 6, p < 0.001).…”

Section: Resultsmentioning

confidence: 98%

“…The attitude of the neurologist is of particular importance because the expected outcome of the proffered MI is a significant determinant of the decision‐making process in ALS 31 . In particular, the decision to opt for mechanical ventilation is based on its potential role in improving QoL (particularly in NIV) and sustaining life (both in NIV and IV), whilst opting against mechanical ventilation is driven by the estimated lack of positive effects on QoL (IV) and the burden imposed on caregivers (both in NIV and IV) 7,32,33 …”

Section: Discussionmentioning

confidence: 99%

“…They include medical interventions (MIs), such as artificial feeding (percutaneous endoscopic gastrostomy, PEG) and breathing support (non‐invasive or invasive ventilation, NIV or IV). If initiated early, they can improve the quality of life (QoL) (PEG and IV) and extend the survival (PEG, NIV, IV), but none of them significantly impacts the ongoing deterioration of physical independence 7,8 . Since life priorities and desires upon the disease outcome differ amongst patients, 9‐11 it is essential for a physician to introduce the patient to both the expected benefits and the risks/consequences of the offered intervention.…”

Section: Introductionmentioning

confidence: 99%

“…If initiated early, they can improve the quality of life (QoL) (PEG and IV) and extend the survival (PEG, NIV, IV), but none of them significantly impacts the ongoing deterioration of physical independence. 7 , 8 Since life priorities and desires upon the disease outcome differ amongst patients, 9 , 10 , 11 it is essential for a physician to introduce the patient to both the expected benefits and the risks/consequences of the offered intervention. Such discussions should start prior to the onset of symptoms, giving enough time for the process of informed consent, therefore, allowing for timely implementation of the measure.…”

Section: Introductionmentioning

confidence: 99%

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Well‐being on supportive techniques in amyotrophic lateral sclerosis: from neurologists' perspective

Barć

Lulé

Finsel

et al. 2022

Ann Clin Transl Neurol

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Objective: To investigate intercultural neurologists' perception of well-being in patients with amyotrophic lateral sclerosis (ALS) using gastrostomy (PEG), non-invasive, and/or invasive ventilation (NIV/IV) and to analyse the determinants and impact on the management of the above medical interventions (MIs). Methods: The study was based on anonymous questionnaires addressing the clinical approach and personal attitude towards the use of PEG, NIV and IV in ALS patients completed by 465 neurologists: 228 from Germany and 237 from Poland. Results: The German and Polish neurologists estimated the quality of life in ALS patients using PEG and NIV as neutral, whilst low in individuals using IV. A regression model revealed an independent influence of palliative care training (PCT) and age on that attitude in the German group. Higher values of estimated patients' depressiveness on PEG, NIV and IV were found amongst the Polish neurologists. Marital status, experience in ALS and being a parent independently influenced the perception of patients' depressiveness in the German, whilst marital status, age and PCT were factors in the Polish group. Amongst German neurologists, a higher perception of patients' depressiveness in individuals using PEG, NIV and IV was linked to the later timing of the MIs discussion. In the Polish group, it was a lower estimation of QoL in patients using PEG. Conclusion: Neurologists' perception of ALS patients' well-being on MIs reflects their demographic status, professional experience and potentially their cultural background. This perception plays an important role in the timing of MIs discussion, possibly influencing the decision-making process.

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Section: Discussionmentioning

confidence: 88%

Section: Resultsmentioning

confidence: 98%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 3 more Smart Citations

Well‐being on supportive techniques in amyotrophic lateral sclerosis: from neurologists' perspective

Barć

Lulé

Finsel

et al. 2022

Ann Clin Transl Neurol

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One third of physicians discuss exit strategies with patients with amyotrophic lateral sclerosis: Results from nationwide surveys among German and Polish neurologists

Barć,

Finsel,

Helczyk

et al. 2024

Brain and Behavior

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ObjectiveThis paper examines neurologists’ approaches to exit strategies (ESs), such as euthanasia and physician‐assisted suicide, in patients with amyotrophic lateral sclerosis (PALS) in two European countries.MethodsIn a nationwide anonymous survey, we collected responses from 237 Polish and 228 German neurologists, focusing on their practices and beliefs about ESs, as well as their viewpoints on life‐sustaining measures (LSMs) (percutaneous endoscopic gastrostomy, non‐invasive, and invasive ventilation). To analyze the data, we employed statistical methods, including Mann–Whitney U, Kruskal–Wallis, chi‐square tests, Spearman's rank correlation, and multiple regression analysis.ResultsOne third of the neurologists initiated the discussion about ESs with PALS. Half were ready to have this conversation upon patient's request. Age, gender, religiousness, and nationality were closely associated with this approach. One in 9 neurologists received a request to terminate an LSM, whereas 1 in 10 to implement an ES. German neurologists and palliative care trainees acquired both demands more commonly. Neurologists quoted a low quality of life, decreased mood, and being a burden to the family/closest ones as primary reasons for a wish to hasten death among PALS. Although the majority expressed a willingness to terminate an LSM at a request of the patient, most opposed the legalization of euthanasia. Younger and less religious individuals were more likely to favor accepting euthanasia.ConclusionNeurologists vary significantly in their approaches to terminal care. Complex relationships exist among personal indices, shared beliefs, and current practices.

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Perry Disease: Expanding the Genetic Basis

Dulski

Koga

Liberski

et al. 2023

Movement Disord Clin Pract

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Background Perry disease (or Perry syndrome [PS]) is a hereditary neurodegenerative disorder inevitably leading to death within few years from onset. All previous cases with pathological confirmation were caused by mutations within the cytoskeleton‐associated protein glycine‐rich (CAP‐Gly) domain of the DCTN1 gene. Objectives This paper presents the first clinicopathological report of PS due to a novel DCTN1 mutation outside the CAP‐Gly domain. Methods Clinical and pathological features of the new variant carrier are compared with another recently deceased PS case with a well‐known pathogenic DCTN1 mutation and other reported cases. Results and Conclusions We report a novel DCTN1 mutation outside the CAP‐Gly domain that we demonstrated to be pathogenic based on clinical and autopsy findings.

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Gastrostomy and mechanical ventilation in amyotrophic lateral sclerosis: how best to support the decision-making process?

Cited by 13 publications

References 118 publications

Well‐being on supportive techniques in amyotrophic lateral sclerosis: from neurologists' perspective

Well‐being on supportive techniques in amyotrophic lateral sclerosis: from neurologists' perspective

One third of physicians discuss exit strategies with patients with amyotrophic lateral sclerosis: Results from nationwide surveys among German and Polish neurologists

Perry Disease: Expanding the Genetic Basis

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